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People with autism and intellectual disabilities, much like individuals with typical development, share a fundamental right and aspiration to realise their own life projects. However, this natural pursuit is uniquely challenging for individuals with autism and intellectual disabilities due to their communication and adaptive hurdles. This growing need has prompted the development of a specific procedure for crafting life projects geared toward enhancing their quality of life. In the present work, we will describe the six key steps and the corresponding assessment, support, and verification tools essential for establishing and actualising the life project for individuals with disabilities, as conceptualised by the Italian Society of Neurodevelopmental Disorders (SIDIN). We will start by delineating diverse preference and value assessment procedures, showcasing an array of tools tailored to accommodate the distinct characteristics of adaptive and communicative functioning in individuals with disabilities. Following this, we will provide a succinct overview of support needs assessment tools. Subsequently, we will introduce the Ecological Life Balance, which serves as an integrative tool for harmonising various assessment systems. We will propose methods for defining existential goals that prioritise quality of life and suggest strategies for implementing support plans. Lastly, we will delve into the methodologies for monitoring and verifying outcomes in the final section.

Objectives: Canakinumab is an IL-1β antibody that neutralises the activity of IL-1β. This study examined the efficacy and safety of canakinumab in patients with moderate COVID-19-related pneumonia., Design: This study aimed to evaluate the reduction in duration of hospitalisation with adequate oxygen status. Forty-eight patients with moderate COVID-19-related pneumonia were asked to participate in the prospective case-control study: 33 patients (cases) signed informed consent and received canakinumab (Cohort 1) and 15 patients (Controls) refused to receive the experimental drug and received institutional standard of care (Cohort 2)., Results: Hospital discharge within 21 days was seen in 63% of patients in Cohort 1 vs. 0% in Cohort 2 (median 14 vs. 26 days, respectively; p < 0.001). There was significant clinical improvement in ventilation regimes following administration of canakinumab compared with Cohort 2 (Stuart-Maxwell test for paired data, p < 0.001). Patients treated with canakinumab experienced a significant increase in PaO 2 :FiO 2 (p < 0.001) and reduction in lung damage by CT (p = 0.01), along with significant decreases in immune/inflammation markers that were not observed in Cohort 2. Only mild side-effects were seen in patients treated with canakinumab; survival at 60 days was 90.0% (95% CI 71.9-96.7) in patients treated with canakinumab and 73.3% (95% CI 43.6-89.1) for Cohort 2., Conclusions: Treatment with canakinumab in patients with COVID-19-related pneumonia rapidly restored normal oxygen status, decreased the need for invasive mechanical ventilation, and was associated with earlier hospital discharge and favourable prognosis versus standard of care., (Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Supporting individuals with NDD is extremely demanding, with significant exposure to critical contexts and events, and painful ongoing experiences. Stress and burnout condition is a main concern with growing interest in research, despite the lack of consensus on theoretical explanatory models and modification standards.The paper provides an up-to-date review of risk factors and involved processes, and presents evidence-based procedures and protocols to implement effective preventive actions addressing both organizational and individual factors. The aim is to offer a global understanding of the subject and offer examples of practical plans to increase the impact on the quality of life of clients and staff members.

For some years, the term "project of life" has become widely used in the field of neurodevelopmental disorders, and, at the same time, it has begun to make its way in many social and health planning documents. However, beyond its relatively widespread use, this term does not yet possess an adequate and shared frame of the main underlying decision-making processes. In particular, there is a need to identify the crucial questions for orienting the choice of goals within the adolescent transition, which represents the complex hinge between childhood and adulthood. Moreover, adulthood, which is often completely devoid of culturally and socially shared references, is still critical precisely because of the lack of future direction prompts usually represented by the stages of development. In this case, the themes of quality of life functioning as a guiding compass appear pertinent and much more relevant. The present contribution is, therefore, an attempt to present, in a unitary manner, the decision-making processes and questions at the basis of a construct of "project of life" shared within the scientific and associative communities.

Background: In the present study a metabolomics-based approach was used to discriminate among different hazelnut cultivars and to trace their geographical origins. Ultra-high-pressure liquid chromatography coupled to quadrupole-time-of-flight mass spectrometry (UHPLC-ESI/QTOF-MS) was used to profile phenolic and sterolic compounds., Results: Compounds were identified against an in-house database using accurate monoisotopic mass and isotopic patterns. The screening approach was designed to discern 15 hazelnut cultivars and to discriminate among the geographical origins of six cultivars from the four main growing regions (Chile, Georgia, Italy, and Turkey). This approach allowed more than 1000 polyphenols and sterols to be annotated. The metabolomics data were elaborated with both unsupervised (hierarchical clustering) and supervised (orthogonal projections to latent structures discriminant analysis, OPLS-DA) statistics. These multivariate statistical tools allowed hazelnut samples to be discriminated, considering both 'cultivar type' and 'geographical origin'. Flavonoids (anthocyanins, flavanols and flavonols - VIP scores 1.34-1.49), phenolic acids (mainly hydroxycinnamics - VIP scores 1.35-1.55) together with cholesterol, ergosterol, and stigmasterol derivatives (VIP scores 1.34-1.49) were the best markers to discriminate samples according to geographical origin., Conclusions: This work illustrates the potential of untargeted profiling of phenolics and sterols based on UHPLC-ESI/QTOF mass spectrometry to discriminate hazelnut and support authenticity and origin. © 2019 Society of Chemical Industry., (© 2019 Society of Chemical Industry.)

Objectives: Ciliary neurotrophic factor (CNTF) is a neurotrophin which could signal neuronal suffering and at the same time acts as a neuroprotective agent. In the present study we aimed to evaluate CNTF serum levels in autism spectrum disorders (ASDs). In fact, considering the role of CNTF as a neuronal damage signal and the role of neuroinflammation, excito-inhibitory imbalance and excitotoxicity in the pathogenesis of ASDs, a possible alteration of CNTF in ASDs could be hypothesised. Methods: We recruited 23 individuals with ASDs and intellectual disability (ID), 20 ID subjects and 26 typical adults. A complete medical and psychopathological characterisation of the participants was performed. CNTF serum levels were measured with ELISA. Results: CNTF serum levels were significantly higher in the ASD + ID group compared to ID ( p  < .001) or typically developed subjects ( p  < .001). Conclusions: CNTF may be considered as a potential biomarker candidate for ASDs in the context of severe ID. Our results support the hypothesis of neurotrophic imbalance in ASDs.

A bioaccessibility study of polyphenols after in vitro simulated large intestine fermentation was carried out on edible nuts. Raw nuts were also analysed for total phenolic content and antioxidant potential, considering both bound and free phenolics. The highest phenolic content was found in walnuts, followed by pistachios extracts (596.9 and 410.1 mg gallic acid equivalents 100 g -1 , respectively). Consistently, the total antioxidant capacity was highest in walnuts (3689.7 μM trolox equivalents 100 g -1 ) followed by peanuts and pistachios (3169.6 and 2990.1 μM trolox equivalents 100 g -1 , respectively). Data showed high correlations between total phenolics and both antioxidant activities. The metabolomics-based phenolic profile depicted during in vitro fermentation showed a degradation of higher-molecular-weight phenolics over 48 hours of faecal fermentation, with a concurrent increase in low-molecular-weight compounds (hydroxybenzoic and hydroxycinnamic acids, alkylphenols, and tyrosols). Our findings indicate that nuts deliver polyphenols into the colon, with bioaccessibility values not negligible for alkylphenols, tyrosols and phenolic acids., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

In this work, an Italian extra-virgin olive oil (EVOO) sample and the same sample added with a carotenoid-rich nutraceutical extract from Lycium barbarum L. (EVOOCar) were subjected to a frying process to comparatively assess chemical and physical changes and heat stability. Oxidation progress was monitored by measuring oil quality changes such as peroxide value, free acidity, K232, K268, and fatty acid composition as well as minor compound content, phenols, α-tocopherol, and carotenoids. An UHPLC/QTOF-MS metabolomics approach discriminated the two oil samples based on their chemical changes during frying, identifying also the phenolic classes most exposed to statistically significant variations. Partial least square discriminant analysis and volcano analysis were applied together to identify the most significant markers allowing group separation. The decrease in total phenolic content was lower in EVOOCar than in EVOO during frying. Monounsaturated and polyunsaturated fatty acids showed a significant percentage loss, 3.7% and 17.2%, respectively, in EVOO after 180min frying at 180°C, while they remained constant or slightly changed in EVOOCar. Zeaxanthin added to the oil rapidly decreased during the frying process. These findings showed that the addition of a carotenoid extract from L. barbarum can help to improve the oxidative stability of extra-virgin olive oil., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Cooking by boiling dry pasta could have varying degrees of influence on nutritional and functional components. In the present study, its effect on total phenolic content and antioxidant capacity, as well as on the comprehensive profile of free and bound phenolics, was investigated in six commercial gluten-free (GF) pasta products. Overall, the heat treatment caused a significant reduction (P<0.01) of the total phenolic content as well as FRAP reducing power and ORAC radical scavenging, with significant differences among the pasta samples considered. The highest values were recorded in free phenolic fraction remaining in black rice (41mggallic acid equivalents100g -1 and 25mmolTrolox Equivalents100g -1 ) and quinoa (24mggallic acid equivalents100g -1 and 14mmolTrolox Equivalents100g -1 ) cooked GF pasta. Significant correlations (P<0.01) could be found between total phenolics and both the antioxidant capacity assays performed. UHPLC-ESI/QTOF-MS mass profiling allowed confirming the spectrophotometric results, while identifying the amount of free and bound fractions. Among phenolic classes, lignans exhibited the highest decrease during the cooking process, followed by stilbenes and flavonoids. However, phenolic acids and other phenolics showed the highest stability. Furthermore, cooking by boiling strongly lowered the bound-to-free ratio of phenolic compounds, by an averaged factor ranging from 14-folds for flavonoids to 5-folds for other classes of phenolics., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

The characterization of phenolic fingerprints in common gluten-free flours is still scarce. Total phenolic and anthocyanin contents, antioxidant capacity and the entire phenolic profile were investigated for extracts from chickpea, sorghum, quinoa, black rice, lentil, amaranth, brown rice, oat and white rice flours, using soft wheat flour as a comparison. The highest phenolic content was found in black rice, followed by quinoa extracts (147.9 and 87.2mg gallic acid equivalents 100g -1 , respectively). Consistently, antioxidant capacity was highest in black rice and quinoa flours (34mmol trolox equivalents 100g -1 ), while anthocyanins were highest in black rice flour. Data showed a high correlation between phenolic content and both reducing and scavenging activities, with Pearson's coefficient of 0.90 and 0.91, respectively. Although the entire phenolic profile was diverse and differed among flours, these represent a valuable source of health-promoting compounds, mainly belonging to flavonoids, phenolic acids and lignans., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

The fate of phenolic compounds, along with short-chain fatty acids (SCFAs) production kinetics, was evaluated on six different commercial gluten-free (GF) pasta samples varying in ingredient compositions, focussing on the in vitro faecal fermentation after the gastrointestinal digestion. A general reduction of both total phenolics and reducing power was observed in all samples, together with a substantial change in phenolic profile over 24h of faecal fermentation, with differences among GF pasta samples. Flavonoids, hydroxycinnamics and lignans degraded over time, with a concurrent increase in low-molecular-weight phenolic acids (hydroxybenzoic acids), alkylphenols, hydroxybenzoketones and tyrosols. Interestingly, discriminant analysis also identified several alkyl derivatives of resorcinol as markers of the changes in phenolic profile during in vitro fermentation. Furthermore, degradation pathways of phenolics by intestinal microbiota have been proposed. Considering the total SCFAs and butyrate production during the in vitro fermentation, different fermentation kinetics were observed among GF pasta post-hydrolysis residues., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Several foods on the market, such as yogurt and fermented milk, include mixtures of prebiotics and probiotic microorganisms effective in promoting the proliferation and equilibrium of intestinal bacteria, thus improving gut health. Particularly, researchers and the public have shown increasing interest in the combination of probiotics with natural substances that promote health or that can act as substrates to promote bacterial growth. The aim of this study is to evaluate the effects of different extracts of Aloe barbadensis and Aloe arborescens in fermented milk, taking into account both the prebiotic effect of aloe polysaccharides and the antimicrobial activity of several secondary metabolites. The results demonstrate a beneficial effect of 5% aloe inner gel on Lactobacillus growth and confirm the antimicrobial activity of the phenolic compounds peculiar of green rind extracts., (© 2016 Institute of Food Technologists®.)

Pure and Na, Ca, and Sr doped lanthanum manganites (LaMnO3, La0.85Na0.15MnO3, La0.7Ca0.3 MnO3, La0.7Sr0.3MnO3) were prepared in form of bulk nano-structured materials through a two-step process. Nanometric powders were synthesized by the Pechini method and subsequently densified by Spark Plasma Sintering at 1273 K for 5 minutes under a pressure of 140 MPa. The method allowed the preparation of single phase manganites with a theoretical density above 98% together with a very limited growth of the grain size. Grain sizes below 70 nm were obtained for all materials except La0.85Na0.15MnO3 which showed a much larger grain size. Curie temperatures (Tc) and magnetoresistivity properties of the samples were measured by a Superconducting Quantum Interference Device (SQUID). The decrease in the resistivity below Tc occurred in a much wider range of temperature compared to ceramics having micron-size grains.

Pure and doped Ba(6)Ti(2)Nb(8)O(30) (BTN), obtained by substituting M = Cr, Mn, or Fe on the Ti site (Ba(6)Ti(2-x) M(x)Nb(8)O(30), x = 0.06 and 0.18) and Y and Fe on the Ba and Ti sites, respectively (Ba(6-x)Y(x)Ti(2-x)Fe(x)Nb(8)O(30), x= 0.18), are synthesized. The influence of cation doping on the local structure, the cation oxidation state, and the possible defect formation able to maintain the charge neutrality are investigated by spectroscopic (electron paramagnetic resonance (EPR) and micro-Raman), structural (X-ray powder diffraction) and transport (impedance spectroscopy, thermoelectric power) measurements, in the temperature range of 300-1200 K in air and N(2) flow. Starting from the valence state of the doping ions (Fe(3+), Cr(3+), and Mn(2+)), determined by EPR, and from thermoelectric power measurements, evidencing a negative charge transport, different charge-compensating defect equilibria, based on the creation of positive electron holes or oxygen vacancies and electrons, are discussed to interpret the conductivity results.

This study deals with the effects of 5 and 10% chromium additions on the transport and structural properties of Li3VO4. The Cr substitution is easily obtained without impurity phases and does not affect the room- and high-temperature host crystal structure, as evidenced by X-ray powder diffraction and micro-Raman analysis. The EPR signals are interpreted in terms of quantified amounts of Cr ions in 5+ and 3+ valence states. Suitable 7Li and 51V MAS NMR spectra simulations agree with the EPR results about the relative amount of Cr5+ and Cr3+ ions substituted in V5+ and Li+ sites, respectively. The Cr3+ presence on Li site, also suggested by Raman results and Rietveld refinements, requires Li vacancies to maintain the charge neutrality. The p-type conductivity, suggested by the positive thermoelectric power coefficients, significantly increases by the cation doping up to an order of magnitude.

Neuroleptic malignant syndrome (NMS), characterized by catatonic behavior, generalized muscular rigidity, hyperthermia and autonomic dysfunction, can suddenly arise in patients treated with neuroleptic agents and carries a high mortality rate even today. A case of NMS due to i.m. therapy with haloperidol decanoate is reported in this paper, characterized by sustained course and a severe relapse due to a different therapy with another neuroleptic agent. The patient however, after a prolonged remission from the relapse, was able to resume long term therapy with neuroleptic drugs. The importance of monitoring blood levels of CPK during the follow-up and the effectiveness of therapy with bromocriptine and dantrolene in confirmed. Although the patient presented a good clinical response, she developed permanent cerebral and muscular damage. A wide review of the literature on NMS is reported to throw light on this syndrome, whose recognition and prompt diagnosis are necessary to reduce mortality.

Two families with hereditary neuropathy with liability to pressure palsies (HNPP) were evaluated clinically, electrophysiologically and pathologically (2 index cases). Familial patterns suggest autosomal dominant inheritance. The clinical presentation is characteristic, with recurrent painless transitory mononeuropathy, without evident triggering factors, or caused by trivial trauma or pressure. In affected members neurophysiological studies showed diffuse slowing in nerve conduction, more evident at common sites of entrapment. Somatosensory evoked potentials (SEPs) showed bilateral peripheral abnormalities with proximal nerve involvement more pronounced in older patients. 40 to 75% of teased fibers displayed sausage-shaped swellings. Ultrastructurally some sausages showed redundant myelin loops and excessive number of myelin lamellae that seem to cause axonal constriction.